Article
Biochemistry & Molecular Biology
Jia Liu, Jingjing Jiang, Jingru Qiu, Liyan Wang, Jing Zhuo, Baozhu Wang, Deqing Sun, Shuyan Yu, Haiyan Lou
Summary: UA, a gut metabolite produced from ellagic acid-containing foods, shows potential protective effects against PD by promoting mitochondrial biogenesis and preventing neurotoxicity induced by 6-OHDA. This study provides new insights into the role of UA in regulating mitochondrial dysfunction in PD and suggests potential therapeutic applications.
Review
Biochemistry & Molecular Biology
Alessandro Luciani, Matthew C. S. Denley, Larissa P. Govers, Vincenzo Sorrentino, D. Sean Froese
Summary: Mitochondria, as the powerhouse of the cell, play a crucial role in sustaining energy metabolism and homeostasis, especially in terminally differentiated cells. Dysregulation of the mitochondrial network can lead to a wide range of hereditary and acquired diseases, highlighting the importance of understanding and potentially reversing the pathophysiology of these disorders.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Materials Science, Multidisciplinary
Mingming Guo, Bowen Li, Guoqing Feng, Xin Su, Xue Wang, Xiaoxuan Han, Manyi Yang, Lipeng Bai, Xiaodong Zhu, Haojun Fan, Bin Zheng
Summary: This study introduces a Co-doped Prussian blue (PB/Co) nanozyme that can scavenge existing ROS and induce mitophagy to eliminate damaged mitochondria. By encapsulating PB/Co nanozyme in two cationic liposomes and developing a non-invasive inhalable nanospray ((PB/Co)@DD), the brain entry efficiency of PB/Co nanozyme is further enhanced. The research shows that (PB/Co)@DD nanospray can induce mitophagy to eliminate ROS production source and restore motor function in PD mice models.
MATERIALS & DESIGN
(2023)
Review
Neurosciences
Iryna Kamienieva, Jerzy Duszynski, Joanna Szczepanowska
Summary: The familial form of Parkinson's disease is linked to mutations in specific genes, with mutations in the parkin gene being one of the most common causes of early-onset PD. Mitochondrial dysfunction is an emerging active player in the pathology of neurodegenerative diseases, as mitochondria are highly dynamic structures integrated with many cellular functions.
TRANSLATIONAL NEURODEGENERATION
(2021)
Review
Cell Biology
Maria Vizziello, Linda Borellini, Giulia Franco, Gianluca Ardolino
Summary: Mitochondrial dysfunction is considered a major pathway in Parkinson's disease, and studies on genetic forms have revealed the roles of PINK1 and Parkin in mitochondrial homeostasis. The PINK1/Parkin pathway mediates the process of mitophagy, which is crucial for maintaining mitochondrial health.
Review
Cell Biology
Wen Li, YuHong Fu, Glenda M. Halliday, Carolyn M. Sue
Summary: Parkinson's disease is an age-related neurodegenerative disorder characterized by the loss of dopaminergic neurons and spread of alpha-synuclein aggregates in the brain. Mitochondrial dysfunction has been identified as a major pathogenic hub for both familial and sporadic PD, playing a key role in disease progression and the formation of Lewy pathology. Therapeutic approaches targeting mitochondrial dysregulation show promise for neuroprotection in PD.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Cell Biology
Elissa Tjahjono, Daniel R. Kirienko, Natalia Kirienko
Summary: Mitochondrial dysfunction is a major cause of various diseases, and surveillance mechanisms play a crucial role in anticipating and repairing damaged mitochondria.
Review
Physiology
Mohamed A. Eldeeb, Rhalena A. Thomas, Mohamed A. Ragheb, Armaan Fallahi, Edward A. Fon
Summary: Mitochondria, as a central hub for cellular metabolism and signaling, play a crucial role in human diseases. Eukaryotic cells have developed sophisticated quality control mechanisms to monitor and repair/mitophagy abnormal proteins and dysfunctional mitochondria. Chaperones help refold unstable proteins, which are selectively degraded if repair is not possible. Autophagy-lysosomal and ubiquitin-proteasome systems mediate the degradation of abnormal proteins. Mitophagy is responsible for eliminating dysfunctional mitochondria to protect cells from damage.
PHYSIOLOGICAL REVIEWS
(2022)
Article
Cell Biology
Xiaowen Ma, Sharon Manley, Hui Qian, Yuan Li, Chen Zhang, Kevin Li, Benjamin Ding, Fengli Guo, Allen Chen, Xing Zhang, Meilian Liu, Meihua Hao, Benjamin Kugler, E. Matthew Morris, John Thyfault, Ling Yang, Hiromi Sesaki, Hong-Min Ni, Heidi McBride, Wen-Xing Ding
Summary: During hepatocyte dedifferentiation, a newly discovered intracellular organelle called the mitochondria-lysosome-related organelle (MLRO) plays a crucial role in regulating mitochondrial homeostasis. The MLRO is formed through the fusion of mitochondria-derived vesicles (MDVs) with lysosomes and is involved in mitochondrial protein degradation and hepatocyte dedifferentiation. This organelle, resembling damaged lysosomes, can be cleared by overexpression of the transcription factor EB (TFEB). The MLRO acts as an alternative mechanism for mitochondrial quality control during cell dedifferentiation, independent of canonical autophagy/mitophagy.
Article
Cell Biology
Fatimah Abd Elghani, Hazem Safory, Haya Hamza, Mor Savyon, Malik Farhoud, Michal Toren-Hershoviz, Zagorka Vitic, Kirsten Ebanks, Vered Shani, Sleman Bisharat, Lihi Shaulov, Claude Brodski, Zhiyin Song, Rina Bandopadhyay, Simone Engelender
Summary: Parkinson's disease (PD) is characterized by the degeneration of dopaminergic neurons in the substantia nigra. In PD, there is an accumulation of alpha-synuclein in Lewy bodies and dysfunctional mitochondria. This study found that SIAH3 protein is increased in the brains and cerebrospinal fluid of PD patients, as well as in neurons treated with alpha-synuclein. SIAH3 interacts with PINK1, leading to their aggregation in mitochondria and triggering toxicity, mitochondrial dysfunction, and neuronal death. The increase in SIAH3 and its aggregation with PINK1 may contribute to alpha-synuclein pathology and prevent the removal of dysfunctional mitochondria.
Article
Biochemistry & Molecular Biology
Yang Song, Chengqun Huang, Jon Sin, Juliana de F. Germano, David J. R. Taylor, Reetu Thakur, Roberta A. Gottlieb, Robert M. Mentzer, Allen M. Andres
Summary: SGLT2 inhibitor empagliflozin can attenuate adverse cardiac remodeling without requiring Parkin to improve cellular energetics. This has potential therapeutic implications for patients with Parkin and mitophagy-related deficiencies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Cell Biology
Sylwia M. Kacprzak, Olivier Van Aken
Summary: This study reveals that carbon starvation induced by dark-incubation, natural senescence, and specific mitochondrial stresses are key triggers of mitophagy in plants.
Article
Multidisciplinary Sciences
Dalila Ciceri, Lierni Gregorio-Zabala, Xabier Llama-Pino, Begum Kurt, Jon Olano-Bringas, Patricia Villegas-Zafra, Nora Bengoa-Vergniory
Summary: Mitochondria play a crucial role in energy metabolism, especially in neurons. Dysfunction of mitochondria is a pathological feature of neurological disorders like Parkinson's disease. The shape and structure of mitochondria can be adjusted based on environmental cues and demands, and it is closely linked to their health. Studying mitochondrial morphology can detect subtle differences in neurodegenerative diseases, such as changes in mitochondrial counts and shape induced by aggregates of Parkinson's disease-related proteins.
JOVE-JOURNAL OF VISUALIZED EXPERIMENTS
(2023)
Article
Medicine, Research & Experimental
Xinyan Li, Mengshen Wang, Su Li, Yuqiong Chen, Mozhi Wang, Zhonghua Wu, Xiangyu Sun, Litong Yao, Haoran Dong, Yongxi Song, Yingying Xu
Summary: The study reveals that high expression of MRPL52 in breast cancer is associated with aggressive clinical characteristics and a higher risk of metastasis. Under hypoxic conditions, MRPL52 promotes migration and invasion of breast cancer cells by regulating autophagy and ROS signaling pathways.
Article
Neurosciences
Kai Yu Ma, Michiel R. Fokkens, Fulvio Reggiori, Muriel Mari, Dineke S. Verbeek
Summary: The p.D620N mutation of VPS35 is associated with mitochondrial dysfunction, affecting the PINK1/Parkin-mediated mitophagy process in PD.
TRANSLATIONAL NEURODEGENERATION
(2021)
Article
Biochemistry & Molecular Biology
Kahori Shiba-Fukushima, Kei-Ichi Ishikawa, Tsuyoshi Inoshita, Nana Izawa, Masashi Takanashi, Shigeto Sato, Osamu Onodera, Wado Akamatsu, Hideyuki Okano, Yuzuru Imai, Nobutaka Hattori
HUMAN MOLECULAR GENETICS
(2017)
Article
Biochemistry & Molecular Biology
Tsuyoshi Inoshita, Taku Arano, Yuka Hosaka, Hongrui Meng, Yujiro Umezaki, Sakiko Kosugi, Takako Morimoto, Masato Koike, Hui-Yun Chang, Yuzuru Imai, Nobutaka Hattori
HUMAN MOLECULAR GENETICS
(2017)
Article
Genetics & Heredity
Yen-Chi Wu, Kyu-Sun Lee, Yan Song, Stephan Gehrke, Bingwei Lu
Review
Genetics & Heredity
Tsuyoshi Inoshita, Changxu Cui, Nobutaka Hattori, Yuzuru Imai
JOURNAL OF GENETICS
(2018)
Article
Biochemistry & Molecular Biology
Masashi Sugo, Hana Kimura, Kohei Arasaki, Toshiki Amemiya, Naohiko Hirota, Naoshi Dohmae, Yuzuru Imai, Tsuyoshi Inoshita, Kahori Shiba-Fukushima, Nobutaka Hattori, Jinglei Cheng, Toyoshi Fujimoto, Yuichi Wakana, Hiroki Inoue, Mitsuo Tagaya
Review
Biochemistry & Molecular Biology
Yuzuru Imai, Hongrui Meng, Kahori Shiba-Fukushima, Nobutaka Hattori
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2019)
Article
Multidisciplinary Sciences
Akio Mori, Taku Hatano, Tsuyoshi Inoshita, Kahori Shiba-Fukushima, Takahiro Koinuma, Hongrui Meng, Shin-ichiro Kubo, Spencer Spratt, Changxu Cui, Chikara Yamashita, Yoshimi Miki, Kei Yamamoto, Tetsuya Hirabayashi, Makoto Murakami, Yoshikazu Takahashi, Hideo Shindou, Takashi Nonaka, Masato Hasegawa, Ayami Okuzumi, Yuzuru Imai, Nobutaka Hattori
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2019)
Article
Biochemistry & Molecular Biology
Aya Ikeda, Kenya Nishioka, Hongrui Meng, Masashi Takanashi, Iwao Hasegawa, Tsuyoshi Inoshita, Kahori Shiba-Fukushima, Yuanzhe Li, Hiroyo Yoshino, Akio Mori, Ayami Okuzumi, Akihiro Yamaguchi, Risa Nonaka, Nana Izawa, Kei-ichi Ishikawa, Hidemoto Saiki, Masayo Morita, Masato Hasegawa, Kazuko Hasegawa, Montasir Elahi, Manabu Funayama, Hideyuki Okano, Wado Akamatsu, Yuzuru Imai, Nobutaka Hattori
HUMAN MOLECULAR GENETICS
(2019)
Article
Multidisciplinary Sciences
Yunpeng Huang, Zhihui Wan, Yinglu Tang, Junxuan Xu, Bretton Laboret, Sree Nallamothu, Chenyu Yang, Boxiang Liu, Rongze Olivia Lu, Bingwei Lu, Juan Feng, Jing Cao, Susan Hayflick, Zhihao Wu, Bing Zhou
Summary: PKAN and PD are two distinct diseases with overlapping pathophysiology. The pathogenic genes PANK2 and PINK1 interact with each other. This study reveals that Fbl (the homolog of PANK2) can rescue PINK1 deficiency by regulating CoA metabolism and mitophagy, providing a potential intervention strategy for PD treatment.
NATURE COMMUNICATIONS
(2022)
Article
Multidisciplinary Sciences
Xingjun Wang, Suman Rimal, Ishaq Tantray, Ji Geng, Sunil Bhurtel, Tejinder Pal Khaket, Wen Li, Zhe Han, Bingwei Lu
Summary: Researchers have discovered that the SARS-CoV-2-encoded Nsp1 can effectively rescue neurodegenerative and behavioral phenotypes in models of Alzheimer's, Parkinson's, and ALS. The Nsp1 acts by resolving collided and stalled translating ribosomes, restoring proteostasis.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Cell Biology
Ji Geng, Tejinder Pal Khaket, Jie Pan, Wen Li, Yan Zhang, Yong Ping, Maria Inmaculada Cobos Sillero, Bingwei Lu
Summary: FMRP interacts with VDAC to regulate the formation and function of ERMCSs, which are critical for mito-Ca2+ homeostasis. Excessive ERMCS formation and ER-to-mitochondria Ca2+ transfer are observed in FXS. Inhibition of VDAC or other ERMCS components restores synaptic structure and function, rescues locomotion and cognitive deficits in FXS models.
DEVELOPMENTAL CELL
(2023)
Article
Biochemistry & Molecular Biology
Suman Rimal, Ishaq Tantray, Yu Li, Tejinder Pal Khaket, Yanping Li, Sunil Bhurtel, Wen Li, Cici Zeng, Bingwei Lu
Summary: The reverse electron transfer (RET) at mitochondrial complex I, which causes increased ROS production and lowered NAD(+)/NADH ratio, is active during aging. Inhibition of RET decreases ROS production, increases NAD(+)/NADH ratio and extends lifespan in flies, highlighting the importance of NAD(+)/NADH rebalance and longevity-associated pathways.
Review
Biochemistry & Molecular Biology
Vishal Chavda, Bingwei Lu
Summary: Stroke is a leading cause of morbidity and mortality globally, with ischemia-reperfusion (IR) injury being a main cause of brain damage. Changes in mitochondrial metabolism during IR lead to the production of reactive oxygen species (ROS) and energy failure. Reverse electron transfer (RET), which redirects electrons derived from succinate, has been implicated in various physiological processes and pathological conditions. This review explores the roles of ROS and RET in the pathogenesis of stroke, as well as their involvement in cancer, aging, and neurodegenerative diseases, providing new insights for targeted therapies.
Article
Biochemistry & Molecular Biology
Keiko Tsuji Wakisaka, Yuzuru Imai
FRONTIERS IN BIOSCIENCE-LANDMARK
(2019)
Article
Biology
Tsuyoshi Inoshita, Nobutaka Hattori, Yuzuru Imai